Pediatric Pleuropulmonary Blastoma Revealed By Massive Hemothorax: Multidisciplinary Diagnostic and Therapeutic Challenges

Pleuropulmonary blastoma (PPB) is a rare pediatric thoracic malignancy that typically presents with nonspecific respiratory symptoms and only exceptionally with spontaneous hemothorax. We report 3 pediatric cases in which PPB initially manifested as severe spontaneous hemothorax: a 2-year-old boy presenting in hemorrhagic shock with right-sided hemothorax (PPB type III), a 3-year-old boy with nontraumatic left hemothorax (PPB type II), and a 3-year-old girl initially suspected of having a vascular malformation who was ultimately diagnosed with PPB type II. In all cases, prompt recognition and a coordinated multidisciplinary approach involving emergency management, surgery, oncology, and intensive care enabled effective treatment. All patients completed therapy and are alive and in good clinical condition at the last follow-up. These cases highlight that, although uncommon, PPB should be included in the differential diagnosis of spontaneous hemothorax in pediatric patients.

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Publication Details

Journal
Journal of Pediatric Hematology/Oncology
Published
2026-04-16
DOI
https://doi.org/10.1097/mph.0000000000003204
Primary Topic
Congenital Diaphragmatic Hernia Studies
Type
article
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article

Pediatric Pleuropulmonary Blastoma Revealed By Massive Hemothorax: Multidisciplinary Diagnostic and Therapeutic Challenges

Paola Francalanci, Giorgio Persano, Angelo Zarfati, Annalisa Serra et al.
Journal of Pediatric Hematology/Oncology
Congenital Diaphragmatic Hernia Studies
article

Pediatric Pleuropulmonary Blastoma Revealed By Massive Hemothorax: Multidisciplinary Diagnostic and Therapeutic Challenges

Paola Francalanci, Giorgio Persano, Angelo Zarfati, Annalisa Serra, Alessandro Crocoli, Cristina Martucci, Chiara Grimaldi, Ilaria Buconi, Gaia Brunetti, Maria D De Paquale, Gian L Natali
article en

Abstract

Pleuropulmonary blastoma (PPB) is a rare pediatric thoracic malignancy that typically presents with nonspecific respiratory symptoms and only exceptionally with spontaneous hemothorax. We report 3 pediatric cases in which PPB initially manifested as severe spontaneous hemothorax: a 2-year-old boy presenting in hemorrhagic shock with right-sided hemothorax (PPB type III), a 3-year-old boy with nontraumatic left hemothorax (PPB type II), and a 3-year-old girl initially suspected of having a vascular malformation who was ultimately diagnosed with PPB type II. In all cases, prompt recognition and a coordinated multidisciplinary approach involving emergency management, surgery, oncology, and intensive care enabled effective treatment. All patients completed therapy and are alive and in good clinical condition at the last follow-up. These cases highlight that, although uncommon, PPB should be included in the differential diagnosis of spontaneous hemothorax in pediatric patients.

Journal of Pediatric Hematology/Oncology
University of Rome Tor Vergata (IT), Anesthesiology and Surgical Oncology Research Group (US), Bambino Gesù Children's Hospital (IT), University Radiology (US)
Good health and well-being
Openalex Percentile: Top 13%
Congenital Diaphragmatic Hernia Studies
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